Friday, March 19, 2010

One Week After Attempted CCSVI Liberation; Oh, What I'd Give to Be Boring...

It seems I've spent my entire life being kind of unconventional, a little bit off-center, a person not wildly eccentric but filled with eccentricities. For the most part, my individuality has served me well, and over time I learned how to capitalize on my somewhat funky qualities to stand apart from the crowd, to separate myself from the writhing masses, and eventually to turn my quirks to my advantage.

Now, medically speaking at least, I find I'd like nothing more than to be a screaming bore, a real snooze, the dullest MS patient on the planet. Unfortunately, my tendency to be atypical has carried over to my disease presentation as well, and while it's garnered my case a lot of attention, that attention has not translated into medical solutions or relief from my illness.

From the get-go, my MS was labeled "atypical". As the years have gone by, more and more eyebrows have been raised by those considering my case, until finally this year the National Institutes of Health decided that I most likely don't have MS. Although my primary neurologist (a happily off-center guy himself) isn't as quick to dismiss MS as a likely culprit, he readily admits that I don't fit the profile of your typical PPMS patient. Unfortunately, the oddities of my case have not worked out to my advantage, as none of the traditional MS treatments have been able to lay a finger on my disease, and the myriad of unconventional remedies I've tried have been utterly useless.

So, I approached the radical CCSVI theory (click here to learn more about CCSVI) in a hopeful but skeptical manner. Even though I don't have classic MS, meaning the CCSVI theory very likely might not apply to me, a CT venogram done early last summer showed an area of stenosis very high up in my internal left jugular vein. Upon seeing this, the NIH opined that my disease may be primarily vascular in nature, but wouldn't commit to that judgment, at least not enough to warrant continued exploration of that possibility.

With no other options left to me, I investigated CCSVI and the idea of a vascular genesis to my illness with great interest, and after many months, with the help of a wily group of determined activist MS patients, found my way to Dr. Salvatore Sclafani, the Interventional Radiologist who performed the Liberation Procedure (a venogram and balloon angioplasty) on me last week. Knowing the peculiarities of my case, Dr. Sclafani and I really weren't expecting to find much in the way of vascular abnormalities during the procedure, but once again, I managed to defy expectations.

Upon injecting dye (via a catheter threaded through my vascular system) into my right internal jugular vein, Dr. Sclafani exclaimed, "Wow!", declared that my vein was significantly blocked, and that I did indeed have the condition now called CCSVI (Chronic Cerebrospinal Venous Insufficiency). Terrific, I thought, at long last a breakthrough!

Dr. Sclafani tried to open the blockage using balloon angioplasty. After four attempts, he had only limited success, the vein was only partially opened and most likely would close back down very soon. He also found some stenosis (narrowing) in my azygos vein, but because of its odd location was unable to fix that, either. Another venogram procedure, using different instruments, would likely be able to fix the azygos problem, but my primary problem appears to be my right jugular vein, which, when compared to the left, looks like something a very drunken Jackson Pollock might have painted on an extremely bad day.

So, the doctor and I are now left with the knowledge that I have some serious Central Nervous System blood flow issues, but are extremely limited in our options for rectifying them. The two most likely options would be traditional "cut the neck open" surgery to remove the malformed valve that is blocking my jugular, or to insert a stent into the vein to press that valve open.

Dr. Sclafani consulted about my case with the doctors and researchers in Italy who have been dealing with CCSVI for several years now, and they commented that they had never seen a valve like mine, that high up in the jugular. Once again, my individuality shines through. Hip hip freaking hooray...

The folks over in Italy suggested that whenever they'd encountered a similar jugular malformation, they'd recommend traditional surgery, or possibly a stent, although they generally try to avoid stents, which are exactly the same conclusions to which Dr. Sclafani had come. Dr. Sclafani talked about my case with some vascular surgeons, who didn't think "open neck" surgery would be a good choice, because of the distinct possibility of severe thrombosis (clotting issues).

This all finally leaves us with the stent option, which I am very uncomfortable with. Although several dozen CCSVI patients have been treated with stents, their use in jugular veins is somewhat controversial, for two primary reasons: stent migration, and eventual stent failure.

It's important to remember that the stents being used were all designed and approved for use in arteries, which are anatomically different from veins in extremely significant ways. A stent that gets loose in an artery generally gets pushed only deeper into the narrowing vessel, and doesn't inflict too much damage. A stent that becomes dislodged in a jugular vein, on the other hand, would inevitably find its way into the heart, with disastrous consequences.

My greater concern, though, is eventual stent failure. The stents now in use were almost all designed and approved to be placed in arteries located in the chest cavity, and these arteries are relatively rigid and not subject to much flexing. The stents now in use are constructed to withstand the tremendous pressures generated by the rhythmic pumping of the heart, not the torque, bending, and twisting they would be subject to when inserted into a vein in the incredibly flexible neck. Laboratory tests have shown that stents do indeed fail over time when subject to the kinds of stresses one might expect them to undergo when placed in very pliant jugular veins. The results of some studies suggest that stents placed under such stresses could likely fracture within 10 years. Needless to say, such a failure could be catastrophic.

Dr. Sclafani, ever the inventive guy, is trying to come up with some alternative solutions, and I'm confident that the good doctor will get it all figured out. In the meantime, though, the choice is between going for the stent, and the inherent dangers that entails, or waiting for the development of a unique procedure that would come with no guarantees, either. And all the while, the relentless drumbeat of my disease progression pounds inexorably onward.

Of course, I'll muster the forces and the gear up for the battles to come, but just this once, it sure would be nice to be scintillating dull...

Yes. Who would think a standard case of MS would be on someone's wish list?

It seems to me that Dr Sclafani loves nothing more than a good puzzle so you've got that on your side. And Dr Big Brain, the NIH, your family, and all of your blog groupies. You really couldn't have more support. My magic wand is still broken so we'll still have to rely on more conventional methods and the collective wishful energy of everyone that is rooting for you.

Hang in there, Marc! Your doctor seems like a great one. You have lots of prayers being sent your way. I also brought up some of the words you spoke to me during our phone interview at my Bible study last night. I think you have been the most inspirational person I have spoken to and your words have really touched me. Good luck on Tuesday-you will be great! I wish I could join Wendy and Tim, but the next time I'm heading to NYC, I'll give you a heads up!

Thanks for sharing your views on the stents. Im am currently mulling over the stent option as my angioplasty is scheduled for March 30 in Poland. I wish those wonderful vascular surgeons out there would hurry up and invent a vein stent! Good luck on your journey!

Very well written Marc. I wish for that touch of boredom and being uninterested but, that's not me (in my mind) physically is a different story! I wonder why a Vascular surgeon would not have all these stent/ballooning and others figured out already. Has no one had a stenosed vein before?

I'm rooting for you to become less unique in your health challenges. Your cognitive abilities seem very sharp at least (mine unfortunately are sloooowing.) It's good to hear Dr. Sclafani contacted the Italians for a discussion about your case (I have a gut feeling he'll find a very good solution for you.) Stay strong---you're not alone even if you're admittedly unconventional.

Good luck, Marc. I don't know if I'd feel better in your case - knowing there is a problem, wondering about solution. Call me da queen of da Nile...;-)In any case, traditional surgery has been done before and if they can get the clotting thing stabilized, could be a partial answer...other stenosed veins, Shirley, are commonly cut out and replaced with healthier ones...or just reattached.Anyway, cheers and best wishes to you, Marc, and I hope a good solution comes soon. Maybe this is why you have atypical MS - and maybe if they can fix these veins, you will be well....hope so, hope so....

Marc, you are far from boring, that's for sure. On the plus side your case has enough interest to grab the attention of some high powered medics. Lets's hope they come up with some viable options soon.

Thanks for the information on stents. CCSVI appears to be a good piece of the MS puzzle. I was really hoping that you would have had some modest positive change. You have gathered a good team of experts who are talking to other experts. You are giving me hope. Aarcyn

Judy-thanks for the thought, but I really don't consider myself a hero. A hero is somebody who runs into a burning building to save somebody else. I'm just trying to save my own ass...

weeble-dammit, I told you to get that magic wand fixed weeks ago. Oh well, your kind thoughts and well wishes are the next best thing...

chelsey-thank you so much for your kind words. The fact that people as sweet as you find me inspirational continues to boggle my mind. In any event, at least it makes my struggle with this disease seem to have some kind of meaning...

Ginger-yes, some new stent designs would be most welcome. In the meantime, when navigating through uncharted waters...

Shirley-as surprising as it sounds, there hasn't been much endovascular surgery done on veins thus far. The primary focus has been on arteries, and veins and arteries are extremely different anatomically.

Patrice-thanks for the well wishes. Dr. Sclafani is indeed a good man, and he has my confidence. This is new for all involved, and the learning process has really only just begun...

Dabble-I keep hoping the atypical nature of my disease might make it more fixable by vascular means also. The NIH seem to think that my problems could be mostly vascular in nature, so who knows. Thanks for your comments, and your support.

Anonymous-I have managed to get myself seen by some of the best minds in the business, unfortunately most of them raise more questions than answers. I'm going to keep trying, though...

Aarcyn-I do believe that CCSVI will be at least a partial piece of the MS puzzle. Getting the doctors to talk amongst each other is a big challenge, each views things through a different set of blinders. I think one of the biggest problems with Western medicine is that doctors have become so specialized that they look at their patients as a bunch of components, not as a whole being...

anonymous-well, it really has yet to be proven just how much stents suck, but I'm not sure I'm willing to let that proof be me.

Anonymous-it could very well be that it takes decades for the damage he done due to irregular blood flow through the CNS to become severe enough to make itself known. It could just be a slow wearing away, like a river slowly carving a canyon...

Marc, Some of the Best things that you write are found in your comments. Thanks for making me laugh. Together we'll get through this and maybe somewhere along the way we can all save our own asses.Charlie

pfrox9-I'll share, I promise. But not too much, because we wouldn't want to get to "normal", it is our quirks that make us interesting...

costume-I can't take the credit for bringing CCSVI to Dr. Sclafani's attention. Another activist patient contacted him and got the ball rolling, and I simply jumped on the wagon pretty early on. I don't want to blow anybody's cover, but credit where credit is due...

Moose-thanks for having my back. Shoot me an e-mail, if you get the chance, I don't have your e-mail address. WheelchairKamikaze@Gmail.com

Hello Marc--I have had MS for almost five years and the last year has proven to be the most challenging because the progression parade has begun. I too am pursuing the CCSVI theory with vigor, but was recently dismayed at the results of my testing. I have perfectly normal veins. No stenosis whatsoever. I had the protocol that was developed by Dr. Mark Haacke, a leader in this field, so I feel the testing was completed correctly. I don't really know what this means at this point in time. It's just a fact I need to mull over. I am truly hoping the CCSVI is a factor in MS progression, and that my case hasn't progressed enough (now walking with cane) for CCSVI to be present. Who knows? One thing I do know though, is the best patient advocates are educated patients. Thanks for being one of them. Regards-Mimi

Marc: Re: your comment about being a hero. I know that what all you are pursuing is for yourself, but taking the time to share it, and so eloquently is a gift to us all. Researchers research generally for their love of the search, yet we all benefit. So maybe hero isn't the right word--how about--you are exceptional in that your reach is wide and you have vision--you see beyond yourself. But I know what you mean about the wince factor of being called courageous, a hero, etc--last night a friend texted me that "I was so strong." Such comments make me fee--well, I feel as if in some ways it is an unconscious demand that I be strong, or at least I feel as if I have to live up to that all the time; I break down all the time--mostly in private--so being called "strong" also makes me feel like a fraud. In my mind I'm very weak--in fact, I feel as if I have no "character." And I also feel the comment has undertones (overtones) of pity. But then again, I want friends to understand the shit storm this is. Am I being too defensive and critical? I know people don't know what to say and that is a better remark than some--as in the friend who is no longer the friend whose response to my news of having MS--"Oh, they have so many treatments for that now. You are so lucky."

Marc,It is with continued admiration I follow your journey. I think that you will be going the stent route as halting the progression and managing the risk of stent detoriation is a more attractive option then the status quo. Any benefits so far from the proceedure? Ruthie and I continue to pray. Warmest regards, Steven Simonyi-Gindele, simonyigindele@yahoo.com

This is such a crazy disease where no two seem to be all that alike. Pretty disappointing to not just be normal, let alone a normal MS victim.

I hope that Dr. Sclafani finds a solution for you. I think these guys really are trying.

Two other things: what a well-written article - kudos! And you're famous - within the CCSVI Calgary group I've heard your name thrown around a couple of times. Thanks for being one of the couragous ones to give it a go, even if it didn't mean a "go" for you.

I love your blog. Do you read any French ? We would be very honoured if you visited our forum at http://www.forseps.org This forum was opened on May 1st in reaction to forces struggling to shut our mouth each time we would pronounce the criminal acronym : CCSVI ! I myself was operated in Sofia (azygous and right jugular blocked to 99%) but did not find a real amelioration, I can still walk but my legs are still very sore, and weak, 100 yards is a maximum for me, EDSS 5. However, I've the "feeling" that my right hand somehow regained in fitness : no keyboard mistakes anymore, which was unstandable to me since I spend half my life writing. Sometimes we have to be aware of very small changes, this was a big one to me. I must go back to Sofia at Christmas. Wish you all the best, keep your happiness 4 us.

I am 4 days post procedure. My left jugular was "functionally absent" and collapsed under the angioplasty. My right one opened up like a charm. I now have 3 stents on the left because there was no risk of losing it - my collateral circulation had been doing the job for years. I am sore and have been placed on coumadin. I am amazingly wakeful in the morning and my color perception is back to normal. I will return to Albany in April...the azygous vein has yet to be explored. Luckily I have a cardiologist for my murmur, my physician would not clear me for research surgery and will not follow me on coumadin. Put your plan in place with a protocol before the procedure.

FYIA young man of Reggio Calabria (south of Italy), with MS and already paralyzed, re-acquired the use of legs after a surgery which consisted in the reconstruction of one of the jugular veins. The surgery was performed by Dr. Salvatore Spagnolo, Head of the Heart Surgery Department of the Policlinico in Monza (Milan, Italy).

MORE INFO ON THE ITALIAN SURGERY HERE:http://www.thisisms.com/ftopicp-154344.html

The story is that a patient in poor condition approached Dr. Spagnolofrom Monza, Italy because had already 3 angioplasty with poor success.He convinced the doctor to do a venogram which showed a totallyblocked IJV.At that point the doctor told the patient that there was nothing he could do.The patient which was relegated to a wheelchair told the doctor he wasconsidering terminating his life because he could not continue likethat.This forced the doctor to attempt a surgical procedure. He took thesaffenous vein from the leg and double its size and use that toreplace the defective Jugular vein. One week later the patient wasable to leave the hospital, this time on his own two feet instead af awheelchair.

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Regretfully, due to the high volume of e-mail received and the realities of living with progressive MS, I'll no longer be able to respond to all e-mails sent. I do read each note, and will do my best to answer as many messages as I can.

About Me

I'm Marc, a 53-year-old male, living in New York City with my lovely and wonderful wife Karen. Diagnosed with Primary Progressive Multiple Sclerosis in March of 2003, I now require a wheelchair to get around the city. I like to drive the wheelchair at full speed, thus the moniker "Wheelchair Kamikaze". I've managed to rig a camera to my chair, so I'm able to take videos and still photos from the unique vantage point of a wheelchair, which I intend to post here.
Before getting sick, I was the Director of DVD Production for one of the major international music companies. Yes, I was once a member of the Evil Empire...
Prior to my enlistment in the Evil Empire, I worked as a video producer and editor.
I grew up in New York City, and spent the 1980s in Boston (college and postcollege rock 'n roll craziness). During the 1990s, I lived in South Florida, until I woke up one morning and realized I was living in South Florida, came to my senses, and moved back to New York.
I hope you like my blog...